Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, has received research and/or training grants from Adamas, Allergan, Biotie, Civitas/Acorda, Hoffmann-La Roche, Medtronic, Merz, Neurocrine , Nuvelution, Pfizer, Prothena, Psyadon, Revance, and Teva; and has served as a consultant or as an advisory committee member for Adamas, Allergan, Merz, Prothena, Revance, and Teva.

)Originally released March 24, 1994; last updated March 9, 2018; expires March 9, 2021

This article includes discussion of Sydenham chorea, minor chorea, poststreptococcal chorea, and rheumatic chorea. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Sydenham chorea is the prototype of chorea resulting from immune mechanisms. Although its incidence has steadily declined in the last decades, it remains the most common cause of acute chorea in childhood worldwide and is still an endemic condition in developing areas of the world. There is also a continuous interest related to the possibility that a similar pathogenic mechanism may be responsible for a subset of patients with tics and other movement disorders, as well as behavioral abnormalities. The author reviews clinical features, pathogenesis, and management of this condition.

Key points

• Sydenham chorea is the most common cause of acute chorea in children worldwide although there is a decline of its incidence.

• Vascular chorea is the most important differential diagnosis of Sydenham chorea.

• Neuropsychiatric symptoms, such as obsessions, compulsions, hyperactivity, and attention disorder, as well as depression, are often present in patients with Sydenham chorea.

• Genetic conditions such as mutations of ACDY5, NKX2-1, PDE10A, and PDE2A may mimic Sydenham chorea.

Historical note and terminology

"Chorea" (derived from the Latin choreus meaning "dance") describes abnormal involuntary movements that are brief, usually distal, and without purpose. First described in the Middle Ages, the most common illness was perhaps a psychogenic movement disorder, but some cases were probably the postinfectious chorea known now as Sydenham chorea. For many years, chorea was the term applied to any hyperkinetic syndrome. Some chorea (including Sydenham chorea) has been called St. Vitus dance (Eftychiadis and Chen 2001). The reader is referred to the Hayden text for an excellent historical review of the various choreic disorders (Hayden 1981) and a review of the “dancing mania” (Krack 1999). Although Thomas Sydenham first described postinfectious choreic movements of children in 1686, the casual relationship of this form of chorea with streptococcal infection was only firmly established 45 years ago (Taranta and Stollerman 1956). There is also evidence indicating that the famous composer Gustav Mahler had Sydenham chorea (Cardoso and Lees 2006). A review of all notes of inpatients seen by Sir William Gowers at the National Hospital of London from 1878 to 1911 showed that almost all chorea cases were caused by Sydenham chorea (Vale et al 2013). The incidence of Sydenham chorea dropped drastically in North America and Western Europe after World War II. Interest in this condition, however, has been fueled lately by its persistence as an important health problem in developing countries, the resurgence of Sydenham chorea in the United States and Australia in the 1990s, the recent outbreaks in Central Europe (Kočevar et al 2017), and the hypothesis that streptococcus-induced antibodies targeted at basal ganglia neurons might account for tics and behavioral abnormalities among children (Ryan et al 2000; Cardoso 2002a; Cardoso 2002c; Cardoso et al 2006c). Sydenham chorea is also known as rheumatic chorea or minor chorea.

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